Splenic B-cell lymphoma/leukemia with prominent nucleoli (SBLPN)
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Based on the current guidelines and the current state of research, there are different diagnostic recommendations for patients with Splenic B-cell Lymphoma/Leukemia with prominent nucleoli. We have summarized the most important information on the classification and diagnostic methods at MLL. In addition, we provide further links on therapy for splenic B-cell lymphoma/leukemia with prominent nucleoli so that you can inform yourself in more detail.
SBLPN: Classification
According to the WHO classification 2022, splenic B-cell lymphoma/leukemia with prominent nucleoli (SBLPN) belongs to the mature B-cell neoplasms and represents a rare disease that shares some cytomorphologic and immunophenotypic features with hairy cell leukemia. The incidence is estimated to be approximately 0.03 cases per 100,000 persons per year (Alaggio et al. 2022). In the previous WHO classification of 2017, SBLPN was named "hairy cell leukemia variant (HZL-v)" (Swerdlow et al. 2017), which is still accepted as an alternative name in the new classification. However, due to pronounced differences and unrelated biology of the two diseases, the new name was introduced. SBLPN is a more aggressive disease without BRAF V600E mutation. It has shorter survival times than HZL and is resistant to conventional hairy cell leukemia therapies (Alaggio et al. 2022).
SBLPN: Diagnostic methods
SBLPN: Prognosis
SBLPN show variable courses that are often more aggressive than those of HZL and are associated with shorter survival times. Median survival is estimated to be approximately 9 years (Alaggio et al. 2022).
SBLPN: Therapy
There is limited information on therapy and response to a number of agents has not been satisfactory. Approaches to treatment strategies are available, for example, from the National Comprehensive Cancer Network (NCCN) and the European Society of Medical Oncology and in the Onkopedia guideline Hairy Cell Leukemia (HZL).
Status: December 2022